Seven children with
steroid resistant nephrotic syndrome (focal segmental
sclerosis in six, mesangial proliferation in one) were treated with
Cyclosporin A for 12 weeks. Five of these children were also resistant to
cyclophosphamide. All patients had normal renal function.
Cyclosporin was started at 8 mg/kg/day then increased until a trough blood level of 100-300 ng/ml (HPLC) was achieved. Three of the seven patients achieved complete remission, and the other four had a significant reduction in their
proteinuria (p less than 0.05). In the three patients who achieved complete remission, relapse of
proteinuria occurred within six weeks of ceasing
Cyclosporin. All patients experienced some impairment in renal function with mean
creatinine clearance decreasing from 129 +/- 19 to 91 +/- 13 ml/min/1.73m2 (p less than 0.05). One child was subsequently treated with
Cyclosporin for 12 months. He remains in remission with a repeat renal biopsy showing no evidence of nephrotoxicity. One other child with
steroid sensitive
minimal change nephrotic syndrome who had severe
steroid toxicity was treated with a lower dose (5 mg/kg/day) for 12 months. She remained in remission off
steroids, but relapsed 16 weeks after
Cyclosporin was ceased. A renal biopsy after 12 months showed no nephrotoxicity.
Cyclosporin should be considered in
steroid resistant nephrotic syndrome, and in children with
minimal change disease who show signs of
steroid toxicity and short remission period after
cyclophosphamide. Serial renal biopsies are recommended with prolonged
therapy.